KM11060 is a mutated F508del cystic fibrosis transmembrane conductance regulator (CFTR) corrector. The most common mutation (~90%) in the CFTR gene is a deletion of phenylalanine 508 (F508del). KM11060 is an analog of sildenafil, which restores a function of the F508del mutated CFTR chloride channel. KM11060 appears to be more potent than sildenafil, forskolin, and genistein.
Chemical Formula: C₁₉H₁₇Cl₂N₃O₂S
Molecular Weight: 422.33
InChi Code: InChI=1S/C19H17Cl2N3O2S/c20-14-1-4-16(5-2-14)27(25,26)24-11-9-23(10-12-24)19-7-8-22-18-13-15(21)3-6-17(18)19/h1-8,13H,9-12H2
Appearance: Solid powder
Purity: >98% (or refer to the Certificate of Analysis)
Shipping Condition: Shipped under ambient temperature as non-hazardous chemical. This product is stable enough for a few weeks during ordinary shipping and time spent in Customs.
Storage Condition: Dry, dark and at 0 - 4 C for short term (days to weeks) or -20 C for long term (months to years).
Solubility: Soluble in DMSO, not in water
Shelf Life: >2 years if stored properly
Drug Formulation: This drug may be formulated in DMSO
Stock Solution Storage: 0 - 4 C for short term (days to weeks), or -20 C for long term (months).
HS Tariff Code: 2934.99.9001
Small-molecule correctors such as KM11060 may serve as useful pharmacological tools in studies of the F508del-CFTR processing defect and in the development of cystic fibrosis therapeutics. KM11060 rescues F508del-CFTR trafficking in cultured cells and native epithelial tissues. KM11060 partially corrects F508del-CFTR processing and increases surface expression to 75% of that observed in cells incubated at low temperature. Up to 50% of the F508del-CFTR in cells treated with KM11060 was complex-glycosylated, indicating passage through the Golgi. KM11060 as a promising compound for further development of CF therapeutics.
In LPS-induced acute lung inflammation, blockade of PSGL-1 (P-selectin glycoprotein ligand-1) or P-selectin, antagonism of PAF by WEB2086, or correction of mutated CFTR trafficking by KM11060 could significantly increase plasma lipoxin A4 levels in F508del relevant to wildtype mice.
Wu H, Yang J, Su EM, Li L, Zhao C, Yang X, Gao Z, Pan M, Sun P, Sun W, Jiang Y, Su X. Lipoxin A4 and platelet activating factor are involved in E. coli or LPS-induced lung inflammation in CFTR-deficient mice. PLoS One. 2014 Mar 26;9(3):e93003. doi: 10.1371/journal.pone.0093003. eCollection 2014. PubMedPMID: 24671173; PubMed Central PMCID: PMC3966846.
Loo TW, Bartlett MC, Shi L, Clarke DM. Corrector-mediated rescue of misprocessed CFTR mutants can be reduced by the P-glycoprotein drug pump. Biochem Pharmacol. 2012 Feb 1;83(3):345-54. doi: 10.1016/j.bcp.2011.11.014. Epub 2011 Nov 28. PubMed PMID: 22138447.
Robert R, Carlile GW, Pavel C, Liu N, Anjos SM, Liao J, Luo Y, Zhang D, Thomas DY, Hanrahan JW. Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect. Mol Pharmacol. 2008 Feb;73(2):478-89. Epub 2007 Nov 1. PubMed PMID: 17975008.
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